Lymphoma, Primary CNS (PCNSL)

	Zambia HIV National Guidelines
	Introduction HIV Counseling and Testing Sexually Transmitted Infections (STIs) General Principles of Antiretroviral Therapy for Chronic HIV Infection in Adults and Adolescents When to Start ARV Therapy for Chronic HIV Infection in Adults and Adolescents Initial Regimen for ARV Therapy Adherence Baseline evaluation and Monitoring Calculations: Ideal Body Weight, Body Mass Index and Creatinine Clearance ARV Therapy for Individuals with Tuberculosis Co-Infection Adverse Effects and Toxicity Immune Reconstitution Inflammatory Syndrome (IRIS) Changing or Stopping ART Treatment Failure Stopping ARV Therapy Post Exposure Prophylaxis Cotrimoxazole Prophylaxis WHO Staging in Adults and Adolescents Nutrition Care and Support Palliative Care in HIV and AIDS
	Guide Editors
	Editor In Chief Joel E. Gallant, MD, MPH Pharmacology Editor Paul Pham, PharmD, BCPS Zambia Guideline Team Peter Mwaba MMed PhD FRCP Alywn Mwinga MMed Isaac Zulu MMed MPH Velepie Mtonga MMed Albert Mwango MBChB Jabbin Mulwanda MMed FCS

Lymphoma, Primary CNS (PCNSL)

Mark Levis, M.D., Ph.D. and Nina Wagner, M.D.
08-17-2009

Zambia Specific Information

Incidence/prevalence of PCNSL in Africa is unknown.
In most studies of HIV-related neuropathology in Africa, PCNSL is rarely reported; cases occasionally reported from S. Africa where more expensive testing modalities more broadly available.
In S. Africa, quantitative PCR for EBV DNA in CSF improves diagnostic specificity, though low positive predictive value precludes its use as an isolated marker for PCNSL.
Dx in Zambia difficult without CSF PCR studies or brain imaging (CT or MRI); primarily a Dx of exclusion made after failure of treatment for CNS toxoplasmosis ± CNS TB.
Prognosis poor, but ART indicated.

Zambia Information Author: David Riedel, M.D.

EBV infection present in virtually 100% of HIV-associated PCNSL
Absence of EBV-specific CD4+ T cell function may provide an immunological basis for HIV-associated PCNSL

Late manifestation of disease, associated with CD4 < 50 (therefore, rarely an AIDS-defining illness).
Incidence declining precipitously since advent of ART.
Presents with variety of focal and non-focal neurological signs and Sx. In contrast to PCNSL in non-AIDS population, is often associated with B Sx.
Histology is immunoblastic, high-grade B cell.
Staging not necessary, as disease remains confined to CNS.

Bx remains gold standard, but not always necessary for Dx. PCR for EBV DNA in CSF helpful in establishing Dx (see below).
Enhancing lesions on CT or MRI (higher yield) generally have DDx of PCNSL vs.CNS toxoplasmosis. Radiographic features characteristic of PCNSL include 1) larger lesions; 2) multifocal lesions; 3) lesions involving corpus callosum; 4) inhomogenous enhancement (reflecting rapid growth rate with necrosis); 5) rarely involve posterior fossa.
Thallium-201 SPECT or PET scans can be used to demonstrate metabolically hyperactive lesion.
CSF cytology for neoplastic cells occasionally positive.
In appropriate clinical setting of low CD4, Toxoplasma seronegativity, large or multifocal hyperenhancing radiographic CNS lesions, and no response to 10-14 days of toxoplasmosis treatment, PCR for EBV DNA in the CSF has high sensitivity and specificity for Dx of PCNSL, often precluding need for Bx.

Prognosis

Outcome data limited in post-ART era, since incidence of HIV-related PCNSL has dropped dramatically.
In absence of ART, med. survival 1-3 mos, and usually not improved with chemotherapy or radiation therapy (XRT).
With ART (and chemotherapy/XRT), overall prognosis still poor (probably 12 to 18 mos), but long-term survival more common.

General considerations

Optimal treatment not yet defined.
Initiation of ART essential for any chance of long-term survival.
High-dose methotrexate (3-8 grams/m² IV with leucovorin rescue beginning 24 hrs later) is current treatment of choice.
Whole brain radiation with concomitant corticosteroids also effective.

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Abbreviations